Girolami Antonio, Fabris Fabrizio, Girolami Bruno
University of Padua Medical School, Department of Medical and Surgical Sciences, Padua, Italy.
Pathophysiol Haemost Thromb. 2002 Sep-Dec;32(5-6):258-62. doi: 10.1159/000073577.
Venous thrombophilia is the result of clotting changes namely of a hypercoagulable state together with blood flow and vessel wall changes. There is no need for all these components to be present in order for thrombosis to occur. As the matter of fact, thrombosis may occur even if only one of these conditions is present. In clinical practice a combination of factors is usualy seen. In comparison with arterial thrombophilia, clotting changes and blood flow seen to play a major role in venous thrombosis. Venous thrombophilia may remain asynptomatic or may result in a series of clinical syndromes. The commonest of these are: 1. Superficial vein thrombosis, 2. Deep vein thrombosis of legs, 3. Deep vein thrombosis of arms, 4. Caval veins thrombosis, 5. Portal vein thrombosis, 6. Hepatic veins thrombosis, 7. Renal vein thrombosis, 8. Cerebral sinuses thrombosis, 9. Right heart thrombosis, 10. Miscellaneous (ovarian, adrenal veins thrombosis, etc.). Since the first two are widely and easily recognized, these is no need for an extensive discussion. Deep vein thromboses of upper limbs are not as frequent as those of lower limbs or of superficial phlebitis but they can still be recognized on clinical grounds and non invasive techniques. The remaining 7 syndromes are less common and therefore less frequently suspected and recognized. Of particular interest, among these less common manifestations of venous thrombophilia are hepatic vein and renal vein thrombosis. Hepatic veins thrombosis, sometimes part of inferior vena cava thrombosis is most frequently due to an isolated occlusion of hepatic veins thereby causing a form of venocclusive disease. Occasionally diagnosis may be difficult because of slow onset of symptoms (hepatomegaly, right flank pain, fever, ascites etc.). The same is true for renal vein thrombosis which may also be of difficult diagnosis since it causes proteinuria and flank pain. The proteinuria is often interpreted as due to a nephrotic syndrome which, incidentally, may cause by its turn renal vein thrombosis. Portal vein thrombosis and cerebral sinuses thrombosis on the contrary are more easily suspected because of ascites, adominal pain, jaundice or headache, eye proptosis, vomiting. Right heart thrombosis should be suspected in cases of recurrent pulmonary embolization. Ovarian or adrenal veins thrombosis are rare. The competent physician should always consider, given certain congenital or acquired conditions, the possibility to be facing a special form of venous thrombosis or a venous thrombosis in unusual sites. An early diagnosis, as often in medicine, is of paramount importance for a prompt treatment and a satisfactory outcome.
静脉血栓形成倾向是凝血变化的结果,即高凝状态以及血流和血管壁变化。血栓形成并不需要所有这些因素都存在。事实上,即使仅存在这些条件中的一个,也可能发生血栓形成。在临床实践中,通常会看到多种因素的组合。与动脉血栓形成倾向相比,凝血变化和血流在静脉血栓形成中似乎起主要作用。静脉血栓形成倾向可能无症状,也可能导致一系列临床综合征。其中最常见的有:1. 浅静脉血栓形成,2. 下肢深静脉血栓形成,3. 上肢深静脉血栓形成,4. 腔静脉血栓形成,5. 门静脉血栓形成,6. 肝静脉血栓形成,7. 肾静脉血栓形成,8. 脑窦血栓形成,9. 右心血栓形成,10. 其他(卵巢、肾上腺静脉血栓形成等)。由于前两种较为常见且易于识别,因此无需进行广泛讨论。上肢深静脉血栓形成不如下肢或浅静脉炎常见,但仍可通过临床依据和非侵入性技术识别。其余7种综合征不太常见,因此较少被怀疑和识别。特别值得关注的是,在这些静脉血栓形成倾向的较不常见表现中,肝静脉和肾静脉血栓形成较为突出。肝静脉血栓形成有时是下腔静脉血栓形成的一部分,最常见的原因是肝静脉孤立性闭塞,从而导致一种形式的静脉闭塞性疾病。偶尔,由于症状出现缓慢(肝肿大、右胁痛、发热、腹水等),诊断可能会很困难。肾静脉血栓形成也是如此,由于它会导致蛋白尿和胁痛,诊断也可能困难。蛋白尿常被解释为由于肾病综合征引起,而肾病综合征反过来又可能导致肾静脉血栓形成。相反,门静脉血栓形成和脑窦血栓形成由于腹水、腹痛、黄疸或头痛、眼球突出、呕吐等症状更容易被怀疑。在反复发生肺栓塞的情况下应怀疑右心血栓形成。卵巢或肾上腺静脉血栓形成很少见。有经验的医生在考虑某些先天性或后天性疾病时,应始终考虑到面临特殊形式的静脉血栓形成或不寻常部位静脉血栓形成的可能性。与医学中常见的情况一样,早期诊断对于及时治疗和取得满意结果至关重要。